How Can I Help a Family with a Special Needs Child Who Needs Help With a Car Seat?

Patient Presentation
A 6-month-old male came to clinic for his health supervision visit. He had generalized hypotonia of unknown cause and was continuing to be evaluated and monitored by pediatric neurology and genetics.
The past medical history showed a full-term male born without complications. Evaluation to date had included negative head imaging, normal creatinine kinase, and genetic evaluation including Prader-Willi testing.
He had been evaluated by the developmental disabilities team and was receiving some physical therapy through the school district’s early intervention program. The physicaltherapist was happy with his progress.
The family history was significant for a paternal cousin who had a child die in infancy of an unknown cause.
The pertinent physical exam a smily, interactive male with growth parameters in the 25-90%.
He had mild but noticeable hypotonia of his trunk. He held is head fairly well once he was placed into a position, but had more problems with transitioning between positions.
He transferred objects but this had a rudimentary quality to it. Cranial nerves, and strength were normal. He had no early primitive reflexes noted.
The rest of his examination including his skin was normal.
The diagnosis of an infant with hypotonia receiving appropriate developmental services was made.
His pediatrician asked how his car seat was fitting as part of his health supervision visit. The parents said that there were no problems currently and they helped support his head with rolled up towels placed to each side as they had been shown to do by a car seat technician.
The pediatrician reminded the parents that he would need to be in the rear-facing car seat until at least 1 year of age and weighed at least 20 pounds.
However, he also told them that because the child also had hypotonia, it might be necessary to keep the child rear-facing to help support his body until an older age and weight.
He said that they would discuss it more at the child’s 12 month health supervision visit and possibly consult with one of the certified car seat technicians that were available through the local police department.

Discussion
Every pediatric health care provider should be familiar with car seat safety basics.
This includes where seats are best located in a vehicle – back seat of the car or closest to the center of the car
What type of seats are available and when can a child use each type:

  • Rear-facing – used for infants < 1 year of age and less than 20 pounds. These have a 3 or 5-point harnesses that hold the child in the car seat. It is safest to be rear-facing as long as possible.
  • Forward facing or convertible car seats that are used forward facing – used by toddlers > 1 year of age and up to approximately 40 pounds. These have a 5-point harnesses that hold the child in the car seat.
  • Booster seat – these are used for preschoolers/school age children who are forward facing. They boost the child upward in the seat but use the vehicles own shoulder and lap belt system to secure the child. A booster seat for children > 40 lbs and 40 inches tall should be used until the behicle seat belt fits well.
  • Vehicle seat belts – used by older school age children and teenagers, generally for children > 80 pounds and > 4 foot 9 inches tall.

Pediatric health care providers should also be familiar with other common venues where child car seats are used such as during shopping or airline flights.
Infant car seats that have lock into shopping carts or infant car seats that are built-in are extremely dangerous and are not recommended. Instead, families should use a stroller or baby backpack during shopping.
Federal Aviation Administration approved car seats are recommended for children up to 4 years of age during airline flights. After age 4, they should be restrained with the lap belt. Booster seats are not allowed.
Car seats should only be used fro travel, not for sleeping, feeding, playing, etc.

Learning Point
Every child should be transported in the safest way possible. Children with special health care needs (CSHCN) need accommodations to travel safely. The specific needs of each child should be evaluated and an appropriate transportation plan made.
This plan needs to be re-evaluated as the child grows and/or if the medical needs change.

Certified car seat technicians can assist healthcare providers and families with problem solving regarding car seat installations and fittings.
Car seat technicians receive special classroom and installation training in using car seat effectively and safely. After initial training, they must continue to update their knowledge and skills through continuing education to maintain their certification.
Local certified car seat technicians can often be found through hospitals, police departments, and various social service agencies. They can also be found through the National Highway Transportation and Safety Administration (NHTSA) website at http://www.nhtsa.dot.gov/people/injury/childps/contacts.

Many local businesses and agencies sponsor car seat inspections. Lists of local inspections can be found at http://www.seatcheck.org or by telephone at 866-SEATCHECK.
The SafeKids Coalition works to improve child safety including car seat safety. Local chapters can be found at http://www.usa.safekids.org/tier2_rl.cfm?folder_id=3120.
Another option is to contact the National Center for the Safe Transportation of Children with Special Health Care Needs at http://www.preventinjury.org or by telephone at 800-620-0143.

General concepts regarding children with special health care needs CSHCN include:

  • The safest place for any child to ride is the back seat. If there are no other options than to transport a CSHCN in the front seat, then the airbag system if present MUST be switched off because of the risk of injury. This may require permission from the NHTSA who can be contacted at http://www.nhtsa.dot.gov or by telephone at 888-327-4236.
  • Children with special health care needs may need special restraint systems (such as a car bed) or need to have modifications made to regular restraint systems (often positioning changes).
    • Special systems need to be evaluated with the help of a certified car seat technician and may also require the services of other professionals such as rehabilitation specialists. To find a local qualified driver rehabilitation specialists, contact a local rehabilitation center or the Association for Driver Rehabilitation Specialists at http://www.driver-ed.org by telephone at 800-290-2344.
    • In certain circumstances, professional transportation such as an ambulance may be necessary (for example a tall child in a cast). Adapted vehicles for family use or use for regular transportation needs such as traveling to school may also be necessary.
    • A car seat and/or harness should never be altered. Even simple alterations can change the safety and performance of car seat. Any contemplated changes should be discussed with a car seat manufacturer.
  • For positioning the child in the center of the seat folded up blankets, towels, or foam can be used. To prevent sliding down into the seat, a smaller cloth like a wash cloth can be rolled up and placed between the child’s bottom and the crotch strap (on a 5 point harness system).
    Nothing should be placed under or behind the actual car seat/restraint.

  • Necessary medical equipement should be secured in the vehicle to prevent it from becoming a projectile in the event of an accident. Car seat technician can also assist with ideas to secure medical equipment.
  • If possible, have a separate person monitor the child in their seat during travel to monitor the child and necessary medical equipment.
  • CSHCN should have a travel pack which includes emergency supplies such as replacement tracheostomies or gastric tubes, 2 times the amount of oxygen and batteries thought necessary for the trip, extra medications, a personal health record to help emergency personnel should there be an accident.

Questions for Further Discussion
1. When does a car safety seat need to be replaced?
2. In what situations can travel vests be used safely?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Information prescriptions for patients can be found at MedlinePlus for these topic: Motor Vehicle Safety.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

American Academy of Pediatrics. Car Safety Seats: A Guide for Families 2008.
Available from the Internet at http://www.aap.org/family/carseatguide.htm (rev. 2008, cited 1/14/2008).

American Academy of Pediatrics. Transporting Children with Special Needs.
Available from the Internet at http://www.aap.org/publiced/BR_SpNeedsCarSeats.htm (rev. 3/2007 , cited 12/13/2007).

American Academy of Pediatrics. Transporting Children with Special Needs.
Available from the Internet at http://www.aap.org/healthtopics/carseatsafety.cfm (cited 1/14/2008).

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Professionalism
    22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.

  • Systems Based Practice
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    Gretchen Vigil, MD
    Associate Professor of Clinical Pediatrics, University of Iowa Children’s Hospital

    Date
    January 28, 2008

  • Can He Use My Toothpaste?

    Patient Presentation
    A 3-year-old male came to clinic for his health supervision visit.
    When discussing dental health the father asked if he should be using adult toothpaste that contained a whitening ingredient.
    The boy had seen a dentist twice, and he and his father were doing toothbrushing twice a day with barely a smear of toothpaste on the brush.
    He had no caries and a fluoridated water supply.
    The pertinent physical exam showed a healthy boy with normal growth and developmental parameters. His oral examination was normal.
    The diagnosis of a healthy child was made.
    The family was counseled that this was probably okay as long as just a smear was used.
    The pediatrician also reminded the family that the child was due for another professional dental examination.
    Later, she did a PubMed literature search and looked at various professional society recommendations but could not find an answer.
    She consulted one of her dental colleagues who gave her a common sense approach which did not recommend a whitening toothpaste but only a fluoride containing toothpaste.
    She then called the family back and updated them on what she had learned.

    Discussion
    Dental health is important to the overall health of children and adults.
    Brushing twice a day with a topical fluoride toothpaste has been showed in meta-analyses of randomized controlled trials to decrease the incidence of dental caries in infants and toddlers.
    Periodic dental evaluation is also important. The first dental appointment is recommended at no later than 6 months after the first tooth eruption and no later than 12 months of age even if no teeth have erupted.

    The first commercially available fluoride-containing toothpaste was Crest® in 1955.
    Since then other ingredients have been added to toothpastes to improve removal of surface stains and improve tooth appearance.
    These include polishing and chemical chelating agents.

    Bleaching agents applied by dental professionals in the dental setting, or prescribed for home use, are common. The whitening agent most commonly used is carbamide peroxide in various concentrations.
    Over the counter whitening agents usually use hydrogen peroxide again in various concentrations. These over the counter whitening agents are usually in the form of whitening strips that are applied to the teeth or whitening gels.
    Studies of whether or not home whitening agents work have depended on the methods used. One study found the whitening strips to be better for improving whiteness than the gels or control.
    Other studies have found whitening strips to cause no changes in the histomorphology or microchemical composition of the matrix, but did have some changes in the fluorescence due to reducing background luminescence.

    The American Academy of Pediatric Dentistry (AAPD) recommends consulting with dentists before judicious use of bleaching for discolored teeth in children and adolescents. Tooth sensitivity and irritation are common side effects to bleaching. Root reabsorption can also occur with bleaching.
    The AAPD also points out that most studies of whitening have only been done in adults.

    Learning Point
    A PubMed literature search and searching of the American Academy of Pediatric Dentistry, American Dental Association and the American Academy of Pediatrics clinical guidelines did not identify a specific answer to what specific type of toothpaste was recommended.
    The AAPD only specifies that a fluoride containing toothpaste should be used.
    A discussion with a pediatric dental colleague provided the following common sense approach:

    • A toothpaste should contain only the ingredients that a child or adult needs or desires. Most children only need the anti-cavity affect that a fluoride toothpaste provides.
      Whitening agents may cause tooth sensitivity and may contain more abrasives that could potentially wear the thinner enamel found on children’s teeth.
      Therefore whitening agents are not recommended.

    • The usual biggest concern with toothpaste is the potential ingestion of excessive fluoride by children, which can be helped by using only a smear/small amount of toothpaste.
    • A toothpaste designed for children is generally recommended because it has a smaller dose of fluoride than adult toothpastes.
    • A children’s toothpaste is often more accepted by children because they have milder flavors also. Mint flavors tend to be too “spicy” or strong for children and they may reject tooth brushing.
      Cinnamon flavors may cause mucosal irritation in children and adults.

    Questions for Further Discussion
    1. What are the indications for fluoride supplementation?
    2. How can pediatric health care providers assist dental professionals to provide dental care to patients?
    3. What causes tooth discoloration?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Child Dental Health and at Pediatric Common Questions, Quick Answers for this topic: Dental Care

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    American Academy of Pediatric Dentistry. Policy on Dental Bleaching for Child and Adolescent Patients.
    Available from the Internet at http://www.aapd.org/media/Policies_Guidelines/P_Bleaching.pdf (rev. 2004, cited 12/13/2007).

    Duschner H, Gˆtz H, White DJ, Kozak KM, Zoladz JR.
    Effects of hydrogen peroxide bleaching strips on tooth surface color, surface microhardness, surface and subsurface ultrastructure, and microchemical (Raman spectroscopic) composition.
    J Clin Dent. 2006;17(3):72-8.

    Garrison GM, Loven B, Kittinger-Aisenberg LG. Clinical inquiries. Can infants/toddlers get enough fluoride through brushing? J Fam Pract. 2007 Sep;56(9):752 – 754.

    Gˆtz H, Duschner H, White DJ, Klukowska MA.
    Effects of elevated hydrogen peroxide ‘strip’ bleaching on surface and subsurface enamel including subsurface histomorphology, micro-chemical composition and fluorescence changes.
    J Dent. 2007 Jun;35(6):457-66. Epub 2007 Mar 6.

    Lo EC, Wong AH, McGrath C.
    A randomized controlled trial of home tooth-whitening products. Am J Dent. 2007 Oct;20(5):315-8.

    American Academy of Pediatric Dentistry. Policy on Periodicity of Examination, Preventive Dental Services, Anticipatory Guidance, and Oral Treatment for Children.
    Available from the Internet at http://www.aapd.org/media/Policies_Guidelines/G_Periodicity.pdf (rev. 2007, cited 12/13/2007).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    6. Information technology to support patient care decisions and patient education is used.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.
    16. Learning of students and other health care professionals is facilitated.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.

  • Systems Based Practice
    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    Date
    January 21, 2008

  • What is the Treatment for Dysfunctional Uterine Bleeding?

    Patient Presentation
    A 15-year-old female came to clinic with a 4 month history of a large amount of bleeding during her periods.
    She had menarche at age 12, with periods lasting between 3-6 days and intervals from 4-6 weeks.
    Her periods became regular around age 13 with duration of 5 days, intervals of 30 days and without excessive bleeding.
    About 7 months ago she had significantly increased her running mileage for cross-country.
    She lost about 5 pounds and during the sports season had menses that were more irregular with intervals of 20 – 35 days and duration of 3-5 days.
    She described the menses flow also as “light to heavy.”After the season was over 3 months ago, she continued to be active with swimming and some running, but markedly cut down on her mileage.
    Since that time her periods continued to be irregular and her last two periods lasted 7-9 days and she had heavier flow necessitating 10-12 pads or more tampons in a day for 4 days at least, and then several lighter flow days.
    She also complained of passing clots during the first 3 days of her period.
    She denied sexual activity currently or in the past. She denied any medications including aspirin or non-steroidal antiinflammatory drugs, complementary or alternative medicines or drugs of abuse.
    The past medical history was negative including excessive bleeding or bruising.
    The family history was negative for gynecological, obstetrical or hematological problems.
    The review of systems was negative.
    The pertinent physical exam showed a thin female with weight of 114 pounds (10-25%) and a height of 68 inches (90%). She had no tachycardia and no orthostasis.
    Skin showed a small bruise on her upper thigh and no hirsutism. She had some closed comedones along her nasal folds and forehead. She had no thyromegaly.
    Genitourinary examination showed a virginal female, Tanner stage V, with normal external genitalia. Pelvic examination performed through the rectum was normal.
    The laboratory evaluation showed a complete blood count with hemoglobin of 11.2 g/dl and hematocrit of 35%, and platelets of 17.5 x 1000/mm2.
    Pregnancy and sexually transmitted infections urine testing were negative.
    Prothrombin time, partial thromboplastin time and thyroid stimulating hormone were normal.
    The diagnosis of dysfunctional uterine bleeding was made. The bleeding was most like due to anovulation secondary to immature hormonal regulation and increased stress and weight loss from her sports activity.
    She had a mild anemia and because she was going to be increasing her sports activities again with track season, it was decided to use oral contraceptive pills to try to regulate her menstrual cycles.
    She was given iron supplementation and was to return to clinic in two months for follow-up and call in the interval if problems became worse.

    Discussion
    Dysfunctional uterine bleeding is prolonged, excessive or frequent, unpatterned uterine bleeding that is not related to an anatomical uterine abnormality or systemic cause.
    It is very common in adolescents and is a clinical diagnosis. The main complication is anemia.

    Menarche occurs from 9-16 years with an average of 12.4 years in the United States. Normal ovulatory cycles can take up to 5 years after menarche to occur.
    Normal menses last 2-7 days, with an interval of 21-35 days and have an upper limit of normal menstrual flow of 60-80 ml.
    Menstruation occurs through the regulation of numerous hormones, primarily estrogen and progesterone, but also luteinizing hormone, follicle stimulating hormone and others.
    Estrogen reaches adult levels fairly early after menarche, usually in the second year.
    Progesterone though may not reach adult levels until 5 years.

    During normal menstruation the follicular phase lasts from the first day of menstrual flow until ovulation or about 14 days. Its primary hormone is estrogen made by ovarian follicles.
    The luteal phase begins with ovulation and ends with menstruation or conception. Its primary hormone is progesterone made by the corpus luteum.
    Because full adult hormonal levels and appropriate regulation may not occur for 5 years, anovulatory cycles (i.e., cycles where the ovarian follicles release an egg and make then make a corpus luteum) occur frequently.
    Therefore, there is estrogen available to grow the uterine endometrial lining, but little or no progesterone to oppose it.
    Unopposed estrogen causes the endometrial lining to outgrow its blood supply causing breakdown and shedding; estrogen also causes decreased vasoconstriction through several mechanisms leading to continued bleeding.
    The result is dysfunctional uterine bleeding.

    Initial laboratory evaluation usually includes a complete blood count, prothrombin time, partial thromboplastin time, pregnancy test and sexually transmitted infection screening.
    If treatment does not alleviate the problem along with careful monitoring then further testing for thyroid stimulating hormone, glucose, prolactin, dehydroepiandrosterone sulfate testosterone, ristocetin factor
    and imaging usually by ultrasound are undertaken.

    Learning Point
    Patients with significant bleeding, acute abdominal pain and associated problems need a comprehensive evaluation possibly including gynecology, surgery and radiology consultations.

    For patients with dysfunctional uterine bleeding that is due to anovulation, treatment varies depending on severity.

    • If the patient has no anemia and is minimally worried, then she can usually be reassured and monitored. A menstrual calendar is useful to record the pattern and symptoms.
    • If the patient has mild anemia (i.e. hemoglobin > 11 g/dl or hematocrit > 33%), then iron supplementation can be used and the patient again monitored while waiting for menstrual normalization.
      If the patient is sexually active or the bleeding is impacting the patient’s quality of life, oral contraception can be used for simultaneous contraception and menstrual normalization.

    • If the patient has moderate anemia (i.e. hemogblobin 9-11 g/dl or hematocrit 27-33%), then low-dose estrogen-progesterone oral contraceptive tablets can be used by taking 2-4 pills every 4-8 hours until the bleeding stops.
      Then the dosage is gradually decreased until 1 tablet/day for 2-3 weeks.
      At that time the tablets are stopped and withdrawal bleeding should occur.
      A cyclic low-dose estrogen-progesterone oral contraception regimen is then begun for an additional 2-3 cycles.
      Iron supplementation should also be used.

    • If the patient has severe anemia (i.e. hemoglobin &lt; 9 g/dl, hematocrit < 27% or dropping parameters), patients need to monitored closely and hemodynamically stabilized as necessary. Oral contraceptives are initiated immediately also. One option is to use 1-2 pills 4 times/day for 4 days, 3 times/day for 4 days, 2 times/day for 2-3 weeks and then allow withdrawal bleeding for 7 days.
      A cyclic low-dose estrogen-progesterone oral contraception regimen is then begun for an additional 3-6 cycles. Iron supplementation should also be used.
      Intravenous estrogen is not usually utilized but may be necessary if there is brisk bleeding.

    Antiemetics may be needed while giving estrogen treatment because estrogen may cause nausea and vomiting.
    Counseling for sexually transmitted infection prevention should also be given to all adolescents as part of health supervision.

    Questions for Further Discussion
    1. What is the differential diagnosis of vaginal bleeding?
    2. When should gynecology be consulted for abnormal uterine bleeding?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Uterine Diseases

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:252-254.

    Aeby AC, Frattarelli LC. Dysfunctional Uterine Bleeding. eMedicine.
    Available from the Internet at http://www.emedicine.com/ped/TOPIC628.HTM (rev. 5/22/06, cited 12/3/07).

    Matytsina LA, Zoloto EV, Sinenko LV, Greydanus DE. Dysfunctional Uterine Bleeding in Adolescents: Concepts of Pathophysiology and Management. Prim Care. 2006 Jun;33(2):503-15.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Professionalism
    22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    January 14, 2008

  • What Are the Long-term Sequelae of Dermatomyositis?

    Patient Presentation
    A 10-year-old female was admitted to the hospital for an evaluation of possible dermatomyositis.
    She first had symptoms 6 months prior to admission when she developed a scaly, red rash on her hands, elbows, knees, bilateral cheeks and eyelids.
    Shortly after this rash appeared she began having stiffness in her hands. She had no fevers, weight loss or other systemic problems.
    She was seen by an adult dermatologist who diagnosed psoriasis. Since that time, despite dermatological treatment, she has not had improvement.
    She needed assistance with activities of daily living at home and at school because of pain and generalized weakness felt to be secondary to deconditioning.
    She was referred to a pediatric rheumatologist who believed the differential diagnosis included psoriatic arthritis, dermatomyositis or an overlapping syndrome with another similar disease process.
    She was begun on prednisone with improvement of the arthritis, however 4 weeks later, her rash was now progressing on her arms, legs and feet and was violaceous around her eyes and upper cheeks.
    She also complained of increasing pain in her right elbow and had difficulty moving it. She said that she also felt weaker.

    The family history is negative for joint, muscular, neurological, kidney or small vessel abnormalities.
    There is a maternal aunt with migraine headaches, another maternal aunt with psoriasis and other family members have eczema.
    Some family members are allergic to penicillin.

    The review of systems is positive for 3 days of abdominal pain that was diffuse and intermittent. She had not had a bowel movement for 3 days and had pebbly stools at baseline.
    She denied blood per rectum. She also has occasional nausea, but no vomiting or acid reflux in her mouth. She has no fever or urinary complaints.
    The pertinent physical exam showed a cooperative girl who had mild pain in her right elbow and no abdominal pain.
    She had growth parameters at ~75%. She as afebrile and had a blood pressure of 115/68.
    She had mild bilateral yellow-white discharge on her eyelids with mild conjunctival injection. Her lips were dry with mild cracking without bleeding.
    Lungs are clear. Cardiac examination is normal with no rubs or murmurs.
    Abdomen was slightly distended but soft with normal bowel sounds. She had stool palpable throughout the colon. She had mild tenderness with deep palpation and no rebound. Stool guaiac was negative.
    There was no organomegaly.
    Skin examination showed a violaceous rash around the eyes and cheeks with mild edema. She also had erythematous, maculopapular patches with mild scaling on the face, arms, legs, chest, abdomen, back and inguinal area. She had golden yellow crusting on some areas of her lower legs.
    Extremity examination showed her right elbow was contracted, red and warm. Additionally, she had full flexion but limited extension of the elbow. She had swollen fingers.
    Neurological evaluation showed cranial nerves and deep tendon reflexes to be normal. She had decreased strength in her neck (4/5), trunk (3/5) and extremities (3/5). She had a normal gait but needed assistance secondary to her generalized weakness.
    An extensive laboratory evaluation was done including a complete blood count showing mild anemia, and increased aldolase, transaminases, lactate dehydrogenase and creatine phosphokinase.
    She had a normal C-reactive protein. All other testing was negative
    The radiologic evaluation included an right elbow radiograph which was normal and a right elbow magnetic resonance that showed mild subcutaneous edema but no effusion.
    Abdominal radiograph showed stool present throughout the colon.
    She also had a magnetic resonance imaging of her pelvis which showed findings within the pelvic musculature consistent with dermatomyositis.
    After the pelvic imaging the rheumatologists felt that diagnosis of dermatomyositis was probable and felt that a muscle biopsy or electromyographic abnormalities was not needed to further confirm the diagnosis.
    The patient’s clinical course was that she was begun on pulse steroid therapy with Solu-Medrol for 3 days by IV infusion for 3 days. Etanercept to be given weekly. She was not started on methotrexate because of her elevated liver function tests.
    She was evaluated by physical and occupational therapy who prescribed range of motion exercises and some accommodations for activities of daily living.
    Her abdominal pain improved after treatment with Miralax® and subsequent bowel movement. the Miralax was continued as prophylaxis for her constipation.
    She was also given lansoprazole and ranitidine for ulcer prophylaxis secondary to her steroids.
    for her impetigo she was treated with clindamycin IV and then changed to oral medication with resolution.
    Ophthalmology consult confirmed conjunctivitis and recommended gentamicin ophthalmic drops because of her immunosuppression and risk of infection. A more comprehensive ophthalmologic evaluation was recommended as an outpatient.
    Her anemia was felt to be secondary to chronic disease.
    Her blood pressure remained normal during admission and did not require treatment.
    At follow-up 10 days later, her rash is slowly resolving and she is having some increased range of motion in her elbow. She continued to be weak however. Her liver function tests were returning to normal and she was to begin methotrexate soon.
    Other laboratory testing for disease monitoring was being completed.

    Figure 57 – Axial T2-weighted (above) and post-contrast T1-weighted (below) MRI images through the level of the hips demonstrate small patchy areas of increased T2 signal and mild enhancement bilaterally and symmetrically in the gluteus muscles and in the subcutaneous tissues of the lateral and posterior thighs. These findings were felt to be compatible with mild dermatomyositis.

    Discussion
    Juvenile dermatomyositis (JDM) is a disease causing inflammation of small vessels in multiple organs. Its etiology is unknown, but possibly is autoimmune in origin. Overall incidence is 2-3/million with females more affected than males.
    Over the past 40 years, there has been a marked improvement in survival (mortality is &lt; 3%) and functionality.

    In one prospective study of JDM patients presenting symptoms, they had: rash (100%, i.e. heliotrope, Groton papules or malar/facial rashes often), weakness (100%), muscle pain (73%), fever (65%), dysphagia (44%), hoarseness (43%), abdominal pain (37%) and arthritis (35%).
    Another study found that JDM patients presenting symptoms had: rash (42-91%), fever (16%), dysphonia (24%), pulmonary problems (11%), arthritis (6%), and gastrointestinal problems (5%).

    Learning Point
    Patients with JDM can have many long-term sequelae.

    • Calcinosis – seen in 22-40% of patients – usually in sites of trauma late in the disease.
    • Gastrointestinal abnormalities – seen in 22-37% of patients – vasculopathy may cause hemorrhage, perforation or ulceration. Esophageal dysmotility, malabsorption and pneumatosis intestinalis have occurred.
    • Growth problems – seen in approximately 30% of patients – these patients were at least 1 standard deviation below their predicted height.
    • Lipodystrophy and metabolic problems – seen in 14-25% of patients – slow, progressive symmetrical loss of subcutaneous fatty tissue that may be generalized, partial or localized. There may be associated metabolic problems including
      acanthosis nigricans, clitoral enlargement, hirsutism, hepatomegaly, insulin resistance, menstrual abnormalities, and hypertriglyceridemia.

    • Nailfold capillary changes – seen in 80-100% of patients – The total number of capillaries may predict overall disease outcome.

    Some patients continue to have long-term problems including weakness (15%), rash (85%), and need for medications (35%). Luckily
    most, if not all, appear to have good educational and vocational outcome with patients attending and finishing school and working.

    Questions for Further Discussion
    1. What medications are available for treatment of dermatomyositis?
    2. What are the diagnostic criteria for dermatomyositis?
    3. What consultants may be necessary for comprehensive care of a child with JDM?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Myositis and Muscle Disorders.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Pachman LM, Hayford JR, Chung A, et. al. Juvenile Dermatomyositis At Diagnosis Clinical Characeristics of 79 Children. J Rheumatology. 1998:25;1198-1204.

    Huber AM, Lang B, LeBlanc CM, et. al. Medium- and Long-term Functional Outcomes in a Multicenter Cohort of Children with Juvenile Dermatomyositis. Arthritis Rheumatology 2000;43:541-49.

    Ramanan AV, Feldman BM. Clinical Outcomes in Juvenile Dermatomyositis. Current Opinion in Rheumatology. 2002;14;658-662.

    Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:854-856.

    Lindsley CB. Juvenile Dermatomyositis Update. Current Rheumatology Reports. 2006:8;174-77.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.

    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement

    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Systems Based Practice
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    Date
    January 7, 2008